Rheumatic heart disease CVS 4 Hisham Alkhalidi

Rheumatic fever Definition Aetiology Pathological changes (Aschoff body) Cardiac and non-cardiac manifestations of rheumatic fever with special emphasis on valvular manifestations (mitral and aortic valves involvement)

group A β-hemolytic streptococcal pharyngitis Rheumatic fever Acute Immunologically mediated Multisystem inflammatory disease Occurs few weeks after an episode of: group A β-hemolytic streptococcal pharyngitis

Rheumatic fever Usually affects children 5-15 years, but first attacks can occur in adults In economically depressed urban areas or developing countries, RF and RHD remain important public health problems

hypersensitivity reaction It appears that the M proteins of certain streptococcal strains induce host antibodies that cross-react with glycoprotein antigens in the heart, joints, and other tissues. Pericarditis is not a big problem as it resolves

Rheumatic fever Acute Lymphocytes and macrophages and plasma cells central necrosis activated macrophages (Anitschkow cells) with prominent nucleoli activated macrophages can also fuse to form giant cells.

Acute rheumatic mitral valvulitis superimposed on chronic rheumatic heart disease. Small vegetations (verrucae 1-2 mm) are visible along the line of closure of the mitral valve leaflet (arrows). Previous episodes of rheumatic valvulitis have caused fibrous thickening and fusion of the chordae tendineae These irregular, warty projections probably arise from the precipitation of fibrin at sites of erosion caused by underlying inflammation and collagen degeneration.

Chronic RHD Mitral stenosis with diffuse fibrous thickening and distortion of the valve leaflets, commissural fusion (arrows) Thickening and shortening of the chordae tendineae CHRONIC leaflet thickening, commissural fusion and shortening, and thickening and fusion of the chordae tendineae Calcification deep in leafelts

Valvular stenosis and regurgitation Chronic RHD Organization of the acute inflammation and subsequent scarring Aschoff bodies are replaced by fibrous scar, so diagnostic forms of these lesions are rarely seen in chronic RHD The major functional consequence of RHD is: Valvular stenosis and regurgitation (stenosis tends to predominate); indeed, RHD is overwhelmingly the most frequent cause of mitral stenosis accounting for 99% of cases

MITRAL VALVE IS THE COMMONEST VALVE AFFECTED MAT 70 % M 25 % M+A With tight mitral stenosis, the left atrium progressively dilates and may harbor mural thrombi. Long-standing congestive changes in the lungs may induce pulmonary vascular and parenchymal changes and in time lead to right ventricular hypertrophy. With pure mitral stenosis, the left ventricle is generally normal. P very rare

Diagnosis of acute RHD serologic evidence of a previous streptococcal infection + two or more of the following Jones criteria: (1) carditis, (2) migratory polyarthritis of the large joints, (3) subcutaneous nodules, (4) erythema marginatum of the skin, and (5) Sydenham chorea, a neurologic disorder with involuntary purposeless, rapid movements One of the Jones criteria manifestations and two minor manifestations (nonspecific signs and symptoms that include fever, arthralgia, or elevated blood levels of acute-phase reactants) Arthritis ....... for a period of days and then subsides spontaneously, leaving no residual disability the carditis include pericardial friction rubs and arrhythmias Myocarditis can be so severe that resulting cardiac dilation causes functional mitral insufficiency and even CHF Myocarditis and arthritis are temporarily

Chronic rheumatic heart disease More likely to occur when the first attack: In early childhood Sever Recurrence The long-term prognosis is highly variable Surgical repair or replacement of diseased valves has greatly improved the outlook for patients with RHD Embolization from mural thrombi, primarily within the atria or their appendages Infective endocarditis superimposed on deformed valves

Chronic RHD The signs and symptoms of valvular disease depend on which valve(s) are involved Mitral stenosis is the most common manifestation Cardiac murmurs Cardiac hypertrophy and dilation CHF Arrhythmias (atrial fibrillation in the setting of mitral stenosis) Thromboembolic complications Increased risk of subsequent infective endocarditis. Left atrial hypertophy -> enlargement and thrombi formation Embolization from mural thrombi, primarily within the atria or their appendages Infective endocarditis superimposed on deformed valves Myocarditis  cardiac dilation  functional mitral valve insufficiency or even congestive heart failure

Endocarditis and pericarditis CVS 5 Hisham Alkhalidi

Lectures 5 Pathology of endocarditis and pericarditis: definitions, classification and causes with emphasis on subacute bacterial endocarditis, marantic endocarditis and chronic constrictive pericarditis

(RHD) is marked by a row of small, warty verrucae along the lines of closure of the valve leaflets IE typically shows large, irregular, and destructive masses that can extend onto the chordae. (NBTE) typically shows small, bland vegetations, usually attached at the line of closure. One or many may be present. Libman-Sacks endocarditis (LSE) has small or medium-sized vegetations on either or both sides of the valve leaflets, or elsewhere on the endocardial surface

Infective endocarditis (IE) A serious infection, characterized by: Microbial invasion of heart valves or mural endocardium Often with destruction of the underlying cardiac tissues Results in bulky, friable vegetations composed of necrotic debris, thrombus, and organisms

IE, Types Acute endocarditis, usually: Subacute endocarditis: Suggests a destructive infection Involvement of a highly virulent organism (staph. Aureus) Attacking a previously normal valve Death within days to weeks in more than 50% of patients despite antibiotics and surgery Subacute endocarditis: Low virulence organisms (strept. Viridans) Colonizing a previously abnormal heart, especially when there are deformed valves The disease typically appears insidiously Follows a protracted course of weeks to months Most patients recover after appropriate antibiotic therapy Acute can lead to abscess Subacute less destructuive

IE Clinical presentation and complications Acute: Fever, rigor, malaise Large vegetation -> emboli: Infarction Metastatic infection affects distant organs like spleen, brain or heart Kidney: Ag-Ab complex -> GN-> nephrotic syndrome or Renal failure Congestive heart failure due to valve disease Can lead to ring abscess and perforation of the aorta and myocardium Death up to 60%

IE Clinical presentation and complications Subacute: Insidious Splenomegaly Non specific fever, weight loss Small vegetations, so less embolic Low mortality

IE Diagnosis is largely made on the basis of positive blood cultures, echocardiographic findings, and other clinical and laboratory findings arrythmia

IE Pathogenesis Bacteremia is a pre-requisite Other organs infection IV drug abuse: Usually Staph aureus, right heart side (Tricuspid) Dental or surgical procedure Trivial injury, skin, gut, urinary bladder Contributory conditions are immunosupression and neutropenia Staph affects both normal and sick valves and account up to 20% of all infection "culture-negative" endocarditis : deep infection, difficult to isolate or previous Rx

IE Favorable conditions of infections Congenital defects Chronic RHD MV Prolapse Deg. Calcific stenosis Bicusped aorta Prosthetic valve (5% in 5 years) Indwelling catheters

Diagnsosis Blood C/S is a major step Duke’s criteria

Nonbacterial Thrombotic Endocarditis NBTE (Marantic endocarditis) Attributed to: Disseminated intravascular coagulation Hypercoagulability Association with malignancy (specially adenocarinoma) Valvular damage is not a prerequisite for NBTE NBTE can be part of Trousseau Endocardial trauma (e.g., from an indwelling catheter) is also a well-recognized predisposing condition

Nonbacterial Thrombotic Endocarditis NBTE (Marantic endocarditis) Gross: loose adherent groups of small nodules on the lines of valve closure (similar to those of acute rheumatic fever), valve leaflets are normal Micro: SMALL, sterile, fibrin and platelets aggregates, no inflammation or fibrosis. Clinically asymptomatic, if large -> may embolize, may become infected Typically mitral valve NON destructive

Endocarditis associated with SLE Unknown etiology Both sides of the valve Deformity to the valve by healing of fibrinoid necrosis and mucoid degeneration Sterile Mitral then tricuspid Antiphopholipid syndrome

aortic and mitral valves are the most common valves affected in IE

Carcinoid heart disease Right side firbosis bioactive products, Tricupsid then pulmonay unless lung carcinoid

Pericarditis Primary vs secondery Acute vs chronic Uremia is the most common systemic disorder associated with pericarditis Isolated pericardial disease is unusual, and pericardial lesions are almost always associated with disease in other portions of the heart or surrounding structures, or are secondary to a systemic disorder. Pericarditis can be due to irradiation Primary mostly viral

Pericarditis Serous Fibrinous, serofibrinous Purulent Hemorrhagic Caseous

Pericarditis Serous: Exudate Non bacterial causes: Rheumatic fever SLE Tumor Uremia Primary viral Serous: CHF, hypoalbuminemia of any cause

Pericarditis Fibrinous, serofibrinous Purulent Same causes of serous Most commonly due to MI Purulent Bacteria (staph, strept, pneuomcocus) Fungi Parasite Can lead to mediastionpericaridtis or congestive pericarditis MI rub Firbinous bread-and-butter pericarditis Purulent can be up to .5 liter

Pericarditis Hemorrhagic: Caseous: Blood + fibrin or pus Surgery TB Tumor Caseous: Fungi Fibrocalcific constrictive pericarditis Serosanguinous: blunt chest trauma, malignancy, ruptured MI, or aortic dissection Chylous: mediastinal lymphatic obstruction

Healing Resolution Fibrosis: Epicardial plaque Thin Thick massive adhesion Less common secondery causes :rheumatic fever, systemic lupus erythematosus, and metastatic malignancies. Pericarditis can (1) cause immediate hemodynamic complications if a significant effusion is present (see below), (2) resolve without significant sequelae, or (3) progress to a chronic fibrosing process.

Adhesive mediastinopericarditis: Constrictive pericarditis: heart contracts against the surrounding structures Constrictive pericarditis: 1 cm thick dense fibrous obliteration Limit diastolic expansion and cardiac output Pump against surrounding lead dilatation and hypertrophy

Chronic Pericarditis: Morphology Ranges from delicates adhesions to dense fibrotic scars that obliterate the pericardial space. In extreme cases the heart can’t expand during diastole : constrictive pericarditis

Clinical picture Atypical chest pain (worse on reclining) High pitch friction rub. Significant exudate cardiac tamponade  faint distant heart sounds, distended neck veins, declining cardiac output and shock Chronic constrictive pericarditis  venous distension and low cardiac output. The consequences of pericardial effusions depend on the ability of the parietal pericardium to stretch. This, in turn, depends on the amount of fluid and the tempo of its accumulation. Thus, slowly accumulating effusions-even as large as 1000 mL-can be tolerated without clinical manifestation. In contrast, rapidly developing collections of as little as 250 mL (e.g., ruptured MI or ruptured aortic dissection) can restrict diastolic cardiac filling to produce fatal cardiac tamponade.